Sarah Friebert

Pediatric Palliative Care Patients: A Prospective Multicenter Cohort Study

OBJECTIVE: To describe demographic and clinical characteristics and outcomes of patients who received hospital-based pediatric palliative care (PPC) consultations. DESIGN, SETTING, AND PATIENTS: Prospective observational cohort study of all patients served by 6 hospital-based PPC teams in the United States and Canada from January to March 2008. RESULTS: There were 515 new (35.7%) or established (64.3%) patients who received care from the 6 programs during the 3-month enrollment interval. Of these, 54.0% were male, and 69.5% were identified as white and 8.1% as Hispanic. Patient age ranged from less than one month (4.7%) to 19 years or older (15.5%). Of the patients, 60.4% lived with both parents, and 72.6% had siblings. The predominant primary clinical conditions were genetic/congenital (40.8%), neuromuscular (39.2%), cancer (19.8%), respiratory (12.8%), and gastrointestinal (10.7%). Most patients had chronic use of some form of medical technology, with gastrostomy tubes (48.5%) being the most common. At the time of consultation, 47.2% of the patients had cognitive impairment; 30.9% of the cohort experienced pain. Patients were receiving many medications (mean: 9.1). During the 12-month follow-up, 30.3% of the cohort died; the median time from consult to death was 107 days. Patients who died within 30 days of cohort entry were more likely to be infants and have cancer or cardiovascular conditions. CONCLUSIONS: PPC teams currently serve a diverse cohort of children and young adults with life-threatening conditions. In contrast to the reported experience of adult-oriented palliative care teams, most PPC patients are alive for more than a year after initiating PPC.

Pediatric Palliative Care and Hospice Care Commitments, Guidelines, and Recommendations

Pediatric palliative care and pediatric hospice care (PPC-PHC) are often essential aspects of medical care for patients who have life-threatening conditions or need end-of-life care. PPC-PHC aims to relieve suffering, improve quality of life, facilitate informed decision-making, and assist in care coordination between clinicians and across sites of care. Core commitments of PPC-PHC include being patient centered and family engaged; respecting and partnering with patients and families; pursuing care that is high quality, readily accessible, and equitable; providing care across the age spectrum and life span, integrated into the continuum of care; ensuring that all clinicians can provide basic palliative care and consult PPC-PHC specialists in a timely manner; and improving care through research and quality improvement efforts. PPC-PHC guidelines and recommendations include ensuring that all large health care organizations serving children with life-threatening conditions have dedicated interdisciplinary PPC-PHC teams, which should develop collaborative relationships between hospital- and community-based teams; that PPC-PHC be provided as integrated multimodal care and practiced as a cornerstone of patient safety and quality for patients with life-threatening conditions; that PPC-PHC teams should facilitate clear, compassionate, and forthright discussions about medical issues and the goals of care and support families, siblings, and health care staff; that PPC-PHC be part of all pediatric education and training curricula, be an active area of research and quality improvement, and exemplify the highest ethical standards; and that PPC-PHC services be supported by financial and regulatory arrangements to ensure access to high-quality PPC-PHC by all patients with life-threatening and life-shortening diseases.

Availability and Use of Palliative Care and End-of-Life Services for Pediatric Oncology Patients

PURPOSE Palliative care prevents or relieves the symptoms caused by life-threatening medical conditions. Previous surveys have shown both underuse and lack of availability of these services for children with cancer throughout North America. We sought to investigate the current practices and resources surrounding palliative and end-of-life care among participating institutions of the Childrens Oncology Group (COG). METHODS A survey regarding practices and resources was developed by the COG palliative care subcommittee and was sent to all 232 institutions to complete for the calendar year 2005. RESULTS The survey was completed by 81% of the institutions. Per institution, there were a mean of 64.6 newly diagnosed patients and 17.7 patients experiencing relapse. A palliative care team was available in 58% of institutions, a pain service in 90%, a hospice in 60%, a psychosocial support team in 80%, and a bereavement program in 59%. Complementary and alternative medicine was available in 39% of institutions and in 95% of the COG institutions community. Most services, even when available, were not well used by patients. CONCLUSION Despite the well-established benefit of pediatric palliative care, it is only offered in 58% of COG institutions caring for children with cancer. In an era where the benefit of palliative care has been clearly established, this number should approach 100%. Efforts should be directed toward understanding barriers to provision of such services, so that they are available and well used at all childhood cancer centers.

Pediatric Palliative Care Programs in Children’s Hospitals: A Cross-Sectional National Survey

BACKGROUND: Pediatric palliative care (PPC) programs facilitate the provision of comprehensive care to seriously ill children. Over the past 10 years many such programs have been initiated by children’s hospitals, but little is known about their number, staff composition, services offered, sources of support, or national distribution. METHODS: In the summer of 2012, we surveyed 226 hospitals as identified by the National Association of Children’s Hospitals and Related Institutions. The survey instrument gathered data about whether their institution had a PPC program, and for hospitals with programs, it asked for a wide range of information including staffing, patient age range, services provided, and financial support. RESULTS: Of the 162 hospitals that provided data (71.7% response rate), 69% reported having a PPC program. The rate of new program creation peaked in 2008, with 12 new programs created that year, and 10 new programs in 2011. Most programs offer only inpatient services, and most only during the work week. The number of consults per year varied substantially across programs, and was positively associated with hospital bed size and number of funded staff members. PPC programs report a high level of dependence on hospital funding. CONCLUSIONS: PPC programs are becoming common in children’s hospitals throughout the United States yet with marked variation in how these programs are staffed, the level of funding for staff effort to provide PPC, and the number of consultations performed annually. Guidelines for PPC team composition, funding, and consultation standards may be warranted to ensure the highest quality of PPC.

Caring for children with advanced cancer Integrating palliative care

The care of children with advanced cancer is multifaceted. Treatment should focus on continued efforts to control the underlying illness whenever possible. At the same time, children and their families should have access to interdisciplinary care aimed at promoting optimal physical, psychological and spiritual wellbeing. Open and compassionate communication can best facilitate meeting the goals of these children and families. However, there remain significant barriers to achieving optimal care related to lack of formal education, reimbursement issues and the emotional impact of caring for a dying child. Future research efforts should focus on ways to enhance communication, symptom management and quality of life for children with advanced cancer and their families. As efforts to break down barriers and create the evidence base continue, we conclude as follows: this is a most rewarding part of the practice of medicine. A kind word and caring attitude are remembered for decades.

Early Integration of Palliative Care for Children with High‐Risk Cancer and Their Families

Despite increasing data to support pediatric palliative care (PPC) as an integral component of high‐quality care for children with life‐threatening conditions and their families, timely integration of PPC is offered inconsistently to children with high‐risk cancer. In this review, we summarize the growing body of literature in support of early integration of PPC for children with high‐risk cancer and their families, advocating that PPC principles and resources are imperative to holistic cancer‐directed care and rooted in evidence‐based medicine. Finally, we offer possible strategies for optimizing integration of PPC into holistic cancer care for children and families.

Research Priorities in Pediatric Palliative Care

OBJECTIVE To synthesize the perspectives of a broad range of pediatric palliative care (PPC) clinicians and parents, to formulate a consensus on prioritization of the PPC research agenda. STUDY DESIGN A 4-round modified Delphi online survey was administered to PPC experts and to parents of children who had received PPC. In round 1, research priorities were generated spontaneously. Rounds 2 and 3 then served as convergence rounds to synthesize priorities. In round 4, participants were asked to rank the research priorities that had reached at least 80% consensus. RESULTS A total of 3093 concepts were spontaneously generated by 170 experts and 72 parents in round 1 (65.8% response rate [RR]). These concepts were thematically organized into 78 priorities and recirculated for round 2 ratings (n = 130; 53.7% RR). Round 3 achieved response stability, with 31 consensus priorities oscillating within 10% of the mode (n = 98; 75.4% RR). Round 4 resulted in consensus recognition of 20 research priorities, which were thematically grouped as decision making, care coordination, symptom management, quality improvement, and education. CONCLUSIONS This modified Delphi survey used professional and parental consensus to identify preeminent PPC research priorities. Attentiveness to these priorities may help direct resources and efforts toward building a formative evidence base. Investigating PPC implementation approaches and outcomes can help improve the quality of care services for children and families.

Pediatric palliative care in the community

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Pediatric bone marrow cellularity: are we expecting too much?

Purpose: Accurate assessment of marrow cellularity is necessary for establishing diagnoses and monitoring the effects of treatment in a large number of malignant and nonmalignant pediatric illnesses, and for evaluating sibling donors for transplantation. However, normal values for age-related bone marrow cellularity in pediatric patients have not been well established. This study was designed to better define pediatric normal values for bone marrow cellularity. Patients and Methods: A retrospective review of 448 bone marrow core biopsy or clot specimens, including 45 samples from healthy donors, were taken from the posterior iliac crest of patients aged from younger than 1 to 18 years (55% male). All samples were collected and fixed in a standardized fashion. Patients with hematopoietic malignancies and other systemic conditions known to impact marrow cellularity were excluded. Results: The mean cellularity of the entire sample was 65.4%. Cellularity was similar in boys and girls, but varied (p < 0.001) with age. Cellularity was highest in patients younger than 2 years (79.8%), and declined in patients 2 to 4 years old (68.6%) and 5 to 9 years old (59.1%). Cellularity remained stable in older patients (60.1% and 61.1%. respectively, in patients 10 to 14 and 15 to 18 years of age). Adjusting for age and gender, mean cellularity was similar in patients with an underlying nonhematologic malignancy compared to healthy donors but was roughly 6% higher in patients with hematopoietic disorders. Conclusions: This study demonstrates that average cellularity during the first two decades of life, using current techniques of marrow collection and standardized analysis, is lower than previously estimated. In addition, cellularity declined with age until the age of 5 years, but was similar thereafter. After adjusting for age. differences according to diagnosis were relatively small.

Pain management for children with cancer at the end of life: Beginning steps toward a standard of care.

T he last few decades of comprehensive cancer treatment through cooperative group trials, together with major advances in supportive care, have resulted in amazing progress in the treatment of childhood cancer. Recent SEER data (Surveillance Epidemiology and End Results) reveals that 5-year relative survival rates for all children ages 0–19 across all sites and all races have increased from 61.7% in 1975 to 80.1% in 2005. At the same time, however, the incidence of cancer across all sites in children ages 0–19 is also increasing, from 12.9 per 100,000 children in 1975 to 17.2 in 2005 [1]. The fourth most common cause of death overall is cancer, which also remains the leading cause of death by disease in children ages 1–19 years [2]. Although the absolute number of children who die in pain is not known, retrospective parent interview studies have shown pediatric oncologists that we are not as skilled at pain management as we report ourselves to be [3,4]. More recent evidence suggests improvement in symptom management for children with cancer [5,6], perhaps due to therapeutic options, education and training, and availability of palliative care programs and services; we, however, still have work to do. Many barriers to adequate pain management exist. In resourcerich countries where opioid availability is generally not the limiting factor (as opposed to resource-poor nations), a few of the reasons for inadequate pain management in the pediatric oncology population include: difficulties with assessment and measurement in young children or those who do not communicate through normal means; poor training for health care professionals in pain management; fear of addiction in patients and/or parents; fear of addiction or criminal prosecution in providers; confusion about use of other medications for children on study protocols; inability to recognize pain by providers or parents; and unwillingness of children or their parents to acknowledge pain for fear of what it signifies (recurrent/relapsed disease, trips back to the hospital, impending death). Despite these and other obstacles, good pain management seems to be the least we can do for our pediatric patients with cancer. In addition to comprising good, holistic pediatric care, adequate pain management improves ability to cope with therapy (both physically and psychologically), improves nutrition and sleep, and facilitates healing. Importantly, for those for whom the journey of cancer will not end in cure, relief of distress from pain at the end of life carries long-lasting implications for bereaved parents, who cope much better when their dying children’s pain is well-controlled [5,7]. Fortunately, recent data indicates some progress in availability, though not necessarily utilization, of pain management and palliative care services for children with cancer. Eighty-one percent of COG institutions responded to a survey about these issues, reporting a median of 45 new diagnoses and 8 deaths in the index year (2005). Pain teams or services were available at 90% of COG institutions, up from 70% in 1998, but were utilized by a median of only one patient per institution with relapsed malignancy; in contrast, palliative care teams were available at only 58%, up from 36% in 1998 [8]. Though assessment and treatment of pain has improved in recent years, as has regulatory attention to the issue, systematic evaluation and response to the problem of pain in children remains understudied. Single institution or health care delivery system initiatives have been undertaken with moderate success and have only rarely focused on patient outcomes rather than on individual performance as a means to implement clinically relevant change [9]. At present, there remains a paucity of studies across hospitals for children with cancer pain at the end of life. To begin to address this issue on a larger scale, Orsey et al. undertook a large, retrospective study of pediatric oncology patients to examine variation in inhospital opioid prescription during their last week of life. In this issue of Pediatric Blood & Cancer, they report on a large cohort of subjects ages 0–24 years who were admitted to and died in 33 children’s hospitals between 2001 and 2005. Noting that opioid narcotics are central to effective pain management for children with end-stage cancer, the investigators employed a single study measure: whether or not eligible patients received daily opioid therapy for 100% of the days of their last week of life spent in the hospital. Using detailed data from the Pediatric Health Information System, the investigators found that of 1,466 eligible subjects hospitalized at time of death, only 56% received opioids every day during the portion of their last week of life for which they were hospitalized. Substantial variation was found across children’s hospitals, ranging from 0% to 90.5% of eligible patients at a given hospital receiving daily opioid therapy. The investigators then sought to determine the impact of hospital-level versus patient-level factors on the data. At the hospital level, there was no significant difference based on the annual volume of children with cancer at the end of life seen at that institution; however, there was a trend toward increased likelihood of use of opioids with more experience (in unadjusted analyses).